1/8. Successful treatment with methylprednisolone pulse therapy for a life-threatening pulmonary insufficiency in a patient with chronic granulomatous disease following pulmonary invasive aspergillosis and Burkholderia cepacia infection.A 14-year-old boy with X-linked chronic granulomatous disease developed severe invasive pulmonary aspergillosis. He was treated with itraconazole and amphotericin b. However, he deteriorated with progressive pulmonary lesions. Burkholderia cepacia was isolated from his bronchoalveolar lavage. Finally, he was given granulocyte transfusions. Following this procedure, his condition rapidly worsened leading to respiratory failure. His lung biopsy demonstrated organizing pneumonia at his right middle lobe. Then, a methylprednisolone pulse therapy was initiated together with the administration of appropriate antibiotics and adequate amounts of amphotericin b. Dramatically, his condition improved. Therefore, a methylprednisolone pulse therapy with appropriate antimicrobial drugs seems to be beneficial for severe pulmonary insufficiency in this type of patients. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/8. Fatal pneumonia caused by Burkholderia cepacia 9 months after resection of aspergilloma.A 69-year-old man developed an episode of severe community-acquired pneumonia 9 months after resection of aspergilloma. Although aspergillus fumigatus was also isolated in the pleural cavity, it did not invade the remaining lung parenchyma. The patient developed progressive bilateral pneumonia leading to death from respiratory failure. Burkholderia cepacia was considered as prime pathogen, as it was repeatedly cultured from sputum and tracheal secretions, as well as the autopsy lung. B. cepacia is resistant to most antibiotics, and seldom causes pneumonia in patients without cystic fibrosis or chronic granulomatous disease. The precise reason that this apparently immunocompetent patient developed B. cepacia pneumonia remains unknown.- - - - - - - - - - ranking = 0.2keywords = granulomatous disease (Clic here for more details about this article) |
3/8. Hemophagocytic syndrome and Burkholderia cepacia splenic microabscesses in a child with chronic granulomatous disease.Hemophagocytic syndrome, splenic microabscesses and pulmonary cavitary lesions were presented in a 17-month-old boy with prolonged fever, hepatosplenomegaly and a history of tuberculous lymphadenitis. Clinical course mimicked tuberculosis. Blood cultures were negative. Ultrasound-guided, percutaneous aspiration from splenic microabscesses grew Burkholderia cepacia. He was treated successfully with trimethoprim-sulfamethoxazole. This child with chronic granulomatous disease had an unusual clinical manifestation of B. cepacia infection.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
4/8. Newly diagnosed chronic granulomatous disease in a 53-year-old woman with crohn disease.BACKGROUND: Chronic granulomatous disease (CGD) is characterized by defective bactericidal activity of white blood cells, specifically, a defect in superoxide production. patients experience infections, predominantly caused by catalase-positive bacteria and fungal organisms, that may be severe and life-threatening. Most cases of CGD are diagnosed in children; however, it may rarely go undiagnosed until adulthood in individuals with unexplained infections and granulomatous inflammation. OBJECTIVE: To describe an adult with crohn disease and recurrent infections who was newly diagnosed as having CGD. methods: A 53-year-old woman with a history of liver abscesses and crohn disease presented with Burkholderia cepacia pneumonia and required a right middle lobe resection. nitroblue tetrazolium test results confirmed the diagnosis of CGD, and Western blot analysis revealed the absence of the 47-phagocyte oxidase protein. Levels of Crohn-associated specific antibodies to saccharomyces cerevisiae and escherichia coli outer membrane porin C were elevated. RESULTS: The patient, newly diagnosed as having CGD, was given intravenous trimethoprim-sulfamethoxazole, after which she improved clinically and was discharged from the hospital in stable condition to receive daily oral trimethoprim-sulfamethoxazole treatment. CONCLUSIONS: The concomitant occurrence of crohn disease and CGD, both characterized by granulomatous inflammation, is noteworthy. This case study demonstrates that CGD should be considered in adults with recurrent infections, especially those caused by catalase-positive organisms, such as B cepacia.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
5/8. burkholderia gladioli osteomyelitis in association with chronic granulomatous disease: case report and review.We describe a case of insidious small bone osteomyelitis and soft tissue abscess with burkholderia gladioli in a 6-year-old Caucasian boy with chronic granulomatous disease. dna sequencing of the 16S ribosomal rna gene confirmed the bacterial identification. Clinical cure was achieved with a combination of antimicrobial therapy and surgical debridement. A review of infections caused by Burkholderia spp., other than burkholderia cepacia complex, in pediatric patients with chronic granulomatous disease is provided.- - - - - - - - - - ranking = 1.2keywords = granulomatous disease (Clic here for more details about this article) |
6/8. Postmortem diagnosis of chronic granulomatous disease: how worthwhile is it?A previously healthy 11 year old boy died unexpectedly after a rapid course of progressive pneumonia. Postmortem microbiology and histopathology suggested an underlying diagnosis of chronic granulomatous disease. This was confirmed by neutrophil oxidative burst and gene mutation analysis of other family members, one of whom benefited from early bone marrow transplantation.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
7/8. Severe Burkholderia (pseudomonas) gladioli infection in chronic granulomatous disease: report of two successfully treated cases.Chronic granulomatous disease (CGD) is characterized by a defect in phagocytic cells that leads to recurrent superficial and deep pyogenic infections. Burkholderia (pseudomonas) gladioli is a gram-negative bacillus in the pseudomallei group of pseudomonads that is known primarily as a plant pathogen. We report two cases of pneumonia, one accompanied by septicemia, caused by B. gladioli in patients with CGD and their successful treatment with antibiotics. We believe these represent the first reports of human disease caused by this organism. We conclude that B. gladioli should be considered a potential pathogen in patients with CGD.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
8/8. Abnormal technetium labelled white cell scan in the colitis of chronic granulomatous disease.A child with colitis was treated for Crohn's disease, diagnosed on history, clinical and colonoscopic findings, radiolabelled white cell bowel scan, and colonic histology. After septicaemia caused by an unusual organism, further investigation lead to a diagnosis of chronic granulomatous disease (CGD). The granulomatous colitis of CGD is clinically, histologically, and on white cell scanning, indistinguishable from that in Crohn's disease and should be considered in atypical cases. infection with unusual 'pseudomonads' should prompt the exclusion of this disorder.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |